A position statement outlining the need for changing the name of diabetes insipidus was co-published in several journals on 14 October 2022.¹ This statement was the product of a global, multi-organisational working group, dedicated to improving patient safety and awareness of the condition amongst non-endocrine health professionals. Miles Levy (Leicester), as a member of this working group, tells us more about the case for this important name change.

WHERE IT ALL STARTED – CROYDON 2008

When Malcolm Prentice, a mild-mannered endocrinologist working in South London, contacted us about the terrible case of Kane Gorny, it was impossible not to be moved or not to think that this must never happen again.

The case had achieved notoriety in the media, as Kane rang 999 from a hospital bed after a routine operation. He was not given water to drink or desmopressin. He told the emergency service on the phone he was dying of thirst on the ward, and he was right. The police arrived only to be turned away by the ward staff, and the following morning Kane died of dehydration. The terrible misunderstanding was that the clinicians in charge thought Kane had diabetes mellitus. In fact, he had diabetes insipidus which, as this readership knows, is a totally different condition.

Rather than the getting fluids and desmopressin he desperately needed, he was getting his blood sugar monitored. Rita Cronin, Kane’s mum, speaks with clarity and dignity about this. We resolved as an endocrine community to sort it out.

CHANGING THE NAME OF A CONDITION

There are very few conditions where the fix is so easy: fluids and desmopressin. It is not so easy, however, to know how to fix complicated healthcare systems. We decided to take a ‘land, sea and air approach’ – from all angles. This has included education (of clinicians, patients and the public), making desmopressin a critical medicine, and succeeding in commissioning a National Confidential Enquiry into Patient Outcome and Death (NCEPOD) enquiry, amongst other things. One blindingly obvious approach was that we should remove the word diabetes from the condition.

There need to be good reasons for name change. There is precedent for this: the rheumatologists got rid of the word Wegner as he was a Nazi. In the case of diabetes insipidus, the reason for change is that the old term is not fit for purpose in modern times. The obesity epidemic has literally swamped any chance of the term diabetes meaning anything other than sugar diabetes; diabetes=mellitus, case closed.

WHAT SHOULD WE CHANGE TO?

This is more difficult. Like naming a child, everyone has their favourite, unconscious bias is at play, and nothing in life is perfect. The sole ambition was always to remove the word diabetes. Names suggested and discarded along the way have included ‘pituitary insipidus’ (etymologically incorrect and not all patients have pituitary disease), ‘ADH deficiency’ (abbreviates to ADHD) and ‘vasopressin deficiency’ (abbreviates to VD – even worse!).

We settled on AVP deficiency (cranial DI) and AVP resistance (nephrogenic DI). The advantage is that it indicates both pathology and treatment in the same breath. Importantly, it was popular with the key players around the globe. We have now had approval for name change from the endocrine societies of the UK, Europe, the USA, Australia, South America, Japan and South East Asia. This is quite an achievement, and we are very proud of it.

Importantly, this could not have been done without patient support groups around the world, who have been our cheerleaders. The Pituitary Foundation has been amazing.

HOW DO YOU OFFICIALLY CHANGE THE NAME OF A CONDITION?

As they say on ‘Bake Off’, it has been a journey. We have had to learn about the worlds of ICD-11 (International Classification of Diseases 11th Revision; https://icd.who.int) and SNOMED CT (Systematized Nomenclature of Medicine – Clinical Terms).

We struck it lucky by finding a helpful influential contact at SNOMED CT. He thinks our case is perfect for name change and is helping us to adopt the new names in 32 countries around the world. For the first few years, we will use AVP-D and AVP-R as synonyms for cranial and nephrogenic diabetes insipidus, whilst keeping the ‘parent’ name DI to allow online searches to catch up, and to enable clinicians to use their preferred term.

Over time, the anticipation is that the new names will be accepted and become standard parlance. Eventually, just as HONK (hyperglycaemic hyperosmolar non-ketotic coma) has become HHS (hyperosmolar hyperglycaemic state) (HONK always sounded silly to me) and bronzed diabetes became haemochromatosis, it is likely that the ‘Artist Formerly Known as Diabetes Insipidus’ will graciously give way to its successors.

WHAT NOW?

We published an article in the August issue of Lancet Diabetes & Endocrinology, reporting an investigation of the perspectives of over 1,000 patients with DI (or whatever it’s now called). Over 85% wanted name change.² Our publication in October of a position statement in the world’s major endocrine journals should raise the profile of this initiative for endocrinologists.¹

The main work is now for us all to shout this from the rooftops. We need your help to do this. We need to educate our fellow doctors, nurses, scientists, patients and the public. We aim to issue press releases in 2023 so that this is covered on TV, radio and social media.

We need to correct the damage done to Kane and his family, and the many others who have come to harm. After all, Kane’s mum Rita has promised me that she’ll be alongside us on the breakfast TV sofa!

Miles Levy
Consultant Endocrinologist
Member of the Working Group for Renaming Diabetes insipidus

REFERENCES

1. Working Group for Renaming Diabetes Insipidus 2022 Endocrine Connections 11 e220378 (also published in Archives of Endocrinology & Metabolism, Clinical Endocrinology, Endocrine Journal, European Journal of Endocrinology, Hormone Research in Paediatrics, Pituitary and Journal of Clinical Endocrinology & Metabolism).
2. Atila C et al. 2022 Lancet Diabetes & Endocrinology 10 P700–P709.